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重型β地中海贫血患者的电生理(脑电图、脑干听觉诱发电位、视觉诱发电位)研究。

Electrophysiological (EEG, BAEP, VEP) study in patients with beta-thalassemia major.

作者信息

Amabile G, Stefano E, Bianco I, Aliquo M C, Giunti P, Pierelli F, Rizzo P A, Morocutti C

机构信息

Istituto di Clinica delle Malattie Nervose e Mentali, Università degli Studi di Roma, La Sapienza.

出版信息

Acta Neurol Belg. 1987 Aug-Oct;87(4):181-90.

PMID:3673492
Abstract

Twenty-four patients affected by beta-thalassemia major were studied by means of combined EEG, VEP and BAEP recordings. All the subjects were treated with regular blood transfusions and chelating therapy (DFO). An elevated incidence of EEG abnormalities (70.8%) consisting of diffused slow waves and/or diffused small sharp spikes was seen. VEP P100 latency was abnormally prolonged in eight patients (33.3%). Furthermore, a voltage increase of N75-P100 (29%) and P100-N145 (33.3%) VEP components was observed. Mean latency and voltage values were significantly increased when compared with those of a control group. No BAEP alterations were observed. No correlations were found between electrophysiological data, serum ferritin levels and transfusional treatment duration. The possible mechanisms involved in provoking such electrophysiological abnormalities are discussed.

摘要

对24例重型β地中海贫血患者进行了脑电图(EEG)、视觉诱发电位(VEP)和脑干听觉诱发电位(BAEP)联合记录研究。所有受试者均接受定期输血和螯合治疗(去铁胺)。发现脑电图异常发生率较高(70.8%),表现为弥漫性慢波和/或弥漫性小棘波。8例患者(33.3%)的VEP P100潜伏期异常延长。此外,还观察到VEP成分N75 - P100(29%)和P100 - N145(33.3%)的电压升高。与对照组相比,平均潜伏期和电压值显著增加。未观察到BAEP改变。在电生理数据、血清铁蛋白水平和输血治疗持续时间之间未发现相关性。文中讨论了引发此类电生理异常的可能机制。

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